It is accepted treatment to give vasopressin to adults in postcardiotomy shock, but such use in children is controversial. Cardiopulmonary bypass is presumed to attenuate the normal endogenous vasopressin response to shock. We hypothesized that levels of vasopressin in children are altered by bypass, and that children having low endogenous levels perioperatively are more likely to develop hypotension, or require vasopressors.

Serial levels of vasopressin were assessed prospectively in children undergoing bypass at a single center.

Of 61 eligible patients, we enrolled 39 (63%). Their median age was 5 months. The mean level of vasopressin prior to bypass was 18.6 picograms per millilitre, with an interquartile range from 2.6 to 11.4. Levels of vasopressin peaked during bypass at 87.1, this being highly significant compared to baseline (p < 0.00005), remained high for 12 hours at a mean of 73.5, again significantly different from baseline (p = 0.002), were falling at 24 hours, with a mean of 28.1 (p = 0.04), and had returned to baseline by 48 hours, when the mean was 7.4 (p = 0.3). Age, gender, and the category for surgical risk had no influence on the levels of vasopressin. There was no statistically significant relationship between the measured levels and hypotension or the requirement for vasopressors, although a few persistently hypotensive patients had high levels subsequent to bypass. Higher levels correlated with higher levels of sodium in the serum (rs = 0.37, p < 0.00005), higher osmolality (rs = 0.37, p < 0.00005), and positive fluid balance (rs = 0.23, p < 0.008). Preoperative use of inhibitors of angiotensin converting enzyme, preoperative congestive cardiac failure, and longer periods of bypass predicted higher levels during the first eight postoperative hours.

Children do not have deficient endogenous levels of vasopressin following bypass, and lower levels are not associated with hypotension. Any therapeutic efficacy of infusion of vasopressin for post-cardiotomy shock in children is likely due to reasons other than physiologic replacement.

To maintain pulmonary valvar function subsequent to repair of tetralogy of Fallot, we have inserted a homograft monocusp when a transjunctional patch was required. In this study, we have evaluated the mid- to long-term outcomes, aiming to determine the durability of the homograft.

Among 218 repairs performed for tetralogy of Fallot between July, 1996, and June, 2005, we inserted homograft monocusps in 54 patients, 4 of whom had associated absent pulmonary valve syndrome, 3 had pulmonary valvar atresia, and 1 had an atrioventricular septal defect with common atrioventricular junction. The median body weight at surgery was 7.8 kilograms, with a range from 3.9 to 42 kilograms. The function of the monocusp valve was assessed by regular echocardiography, using the Kaplan-Meier method and the Cox regression model for statistical analyses.

There were 2 early deaths (3.7%), associated with respiratory infection. No late deaths were observed during the follow-up, which ranged from 0.3 to 120 months, with a median of 64.3 months. Freedom from valvar dysfunction was 67.2 ± 6.7% at 1 year, 37.1 ± 7.3% at 3 years, 23.8 ± 6.7% at 5 years, and 21.2 ± 6.4% at 7 years. We needed to replace the valve in 1 patient during follow-up. We found that ABO blood group incompatibility, stenosis of the pulmonary arteries, and associated absent pulmonary valve syndrome all adversely affected the function of the monocusp.

Our experiences show that insertion of a homograft monocusp can prevent pulmonary regurgitation in the early period after repair of tetralogy of Fallot, but the effects are limited in duration as degeneration progressed. We still need to determine whether this finding can improve the longer-term function of the right ventricle.

Some centres have proposed creating the bidirectional cavopulmonary anastomosis without cardiopulmonary bypass, while others continue to use deep hypothermic circulatory arrest. The purpose of this review is to evaluate the results of using continuous cardiopulmonary bypass with moderate hypothermia, perhaps the most commonly used of the three techniques for this procedure.

Between 1990 and 2005, 114 patients, having a mean age of 1.58 years, with a median age of 8 months, and ranging from 3 months to 16 years, underwent creation of either a unilateral cavopulmonary anastomosis, in 94 cases, or bilateral anastomoses in 20 cases. All had continuous cardiopulmonary bypass with moderate hypothermia at 32 degrees Celsius, with 24 also having aortic cross-clamping with cardioplegia for simultaneous intracardiac procedures. Interrupted absorbable sutures were used to create the anastomosis in 105 patients.

Perioperative mortality was 5%, with 6 of the patients dying. The mean period of cardiopulmonary bypass for an isolated anastomosis was 91 minutes, with a range from 44 to 160 minutes. In 10 patients (8.8%), it was necessary to place a graft to augment the anastomosis. The average postoperative length of stay was 7.9 days for those undergoing an isolated unilateral anastomosis, and 16.4 days for patients undergoing combined cardiac operations. We have now created the Fontan circulation in 79 of the patients, at an average interval from the bidirectional cavopulmonary anastomosis of 2.1 plus or minus 1.14 years. In 76 patients, we performed postoperative angiograms, and none revealed any stenoses.

The bidirectional cavopulmonary anastomosis can be performed successfully with continuous cardiopulmonary bypass and moderate hypothermia with a beating heart, avoiding circulatory arrest. The use of interrupted and absorbable sutures was not associated with any late anastomotic stenosis.

The policy of early repair of patients with tetralogy of Fallot, irrespective of age, as opposed to initial palliation with a shunt, remains controversial. The aim of our study was to analyze the midterm outcome of primary early correction of tetralogy of Fallot.

Between 1996 and 2005, a total of 61 consecutive patients less than 6 months of age underwent primary correction of tetralogy of Fallot in two institutions. The median age at surgery was 3.3 months, and 27 patients (44%) were younger than 3 months of age, including 12 (20%) newborns. We analyzed the patients in 2 groups: those younger than 3 months of age, and those aged between 3 and 6 months.

There was one early (1.6%), and one late death. Actuarial survival was 98.4%, 96.7%, 96.7% at 1, 5, and 10 years respectively, with a median follow up of 4.5 years. There was no difference in survival, bypass time, lengths of ventilation, and hospital stay between the groups. A transjunctional patch was placed significantly more often in the patients younger than 3 months (p = 0.039), with no adverse effect on survival and morbidity during the follow-up. Freedom from reoperation was 98.2%, 92.2%, and 83% at 1, 5, and 10 years respectively, with no difference between the groups.

Elective primary repair of tetralogy of Fallot in asymptomatic patients is delayed beyond 3 months of age. In symptomatic patients, primary repair of tetralogy of Fallot is performed irrespective of age, weight and preoperative state. This approach is safe, and provides an excellent midterm outcome with acceptable morbidity and rates of reintervention. The long-term benefits of this approach must be established by careful follow-up, with particular emphasis on arrhythmias, right ventricular function, and exercise performance.

Stenoses in the pulmonary arterial system can have a significant negative impact on the early postoperative course in infants. Early recognition and aggressive management are mandatory.

We describe our experience with 8 infants, with ages ranging from 3 to 9 months, weighing from 4.5 to 7.7 kilograms, who presented in the up to 18 days following construction of a shunt from the superior caval vein to the pulmonary arteries with clinical symptoms of obstructed pulmonary flow. We include also 2 infants in whom pulmonary arterial stents were implanted in the operating room. Cardiac catheterization showed significant stenoses or occlusion of the left pulmonary arteries in 9 infants, the right pulmonary arteries in 2, or the superior caval vein in 1, the investigation being prompted by the findings of supraphysiological superior caval venous pressures and systemic hypoxaemia. We implanted a variety of stents mounted on balloons ranging in diameter from 6 to 13 millimetres, with 7 placed across a newly created surgical anastomotic site.

All stenoses were crossed successfully, and stents implanted satisfactorily in all patients, albeit that 1 infant suffered an acute tear of the left pulmonary artery, requiring immediate reoperation. This patient died 72 hours later due to a diffuse coagulopathy. All other patients demonstrated sustained clinical improvement following the procedure. At follow-up, 7 of the 9 survivors have progressed to completion of the Fontan circulation. Redilation of the stents was required in the interim, prior to completion of the Fontan circulation, in 4 of them. In 2 patients, the previously implanted stents were incised during the Fontan completion, permitting placement of the extracardiac Goretex conduit from the inferior caval vein to the pulmonary arteries.

Stents can successfully be implanted perioperatively in the pulmonary arterial system during infancy, and redilated, with improvement in clinical outcome in the majority of those with clinically relevant obstruction.

Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous artery arises from the right posterior pulmonary sinus. We report a new technique for re-implantation using combined autogenous aortic and pulmonary arterial flaps in situations when a direct connection was not possible.

We have treated 4 patients, aged 3 months, 6 months, 18 months, and 27 years respectively, who presented with anomalous origin of the left coronary artery from the left posterior pulmonary sinus. We used our proposed technique for transfer because lack of coronary arterial length, diminished vessel elasticity, and extensive collaterals around the pulmonary sinuses prevented direct attachment.

There was no early or late death. Postoperatively, all patients are in functional class I, with good biventricular function at a median follow-up of 74 months, with a range from 9 to 96 months. Postoperative coronary angiography in our 4th patient showed good arterial flow, without any distortion.

The potential benefits of this modification of the trapdoor technique are excellent operative exposure, use of autogenous and viable tissue capable of further growth, avoidance of injury to the aortic and pulmonary valvar apparatus and production of obstruction within the right ventricular outflow tract, complete elimination of use of pericardium for augmentation of the neo-aortic tube, achievement of the anastomosis with correct angling and length, and the possibility of implantation in all patients, including adults, regardless of the distance from the aorta or the coronary arterial configuration.